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König, Nicole ; Stetefeld, Henning R. ; Dohmen, Christian ; Mergenthaler, Philipp ; Kohler, Siegfried ; Schönenberger, Silvia ; Bösel, Julian ; Lee, De-Hyung ; Gerner, Stefan T. ; Huttner, Hagen B. ; Schneider, Hauke ; Reichmann, Heinz ; Fuhrer, Hannah ; Berger, Benjamin ; Zinke, Jan ; Alberty, Anke ; Kleiter, Ingo ; Schneider-Gold, Christiane ; Roth, Christian ; Dunkel, Juliane ; Steinbrecher, Andreas ; Thieme, Andrea ; Schlachetzki, Felix ; Linker, Ralf A. ; Angstwurm, Klemens ; Meisel, Andreas ; Neumann, Bernhard

MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation

König, Nicole, Stetefeld, Henning R., Dohmen, Christian, Mergenthaler, Philipp, Kohler, Siegfried, Schönenberger, Silvia, Bösel, Julian, Lee, De-Hyung, Gerner, Stefan T. , Huttner, Hagen B., Schneider, Hauke , Reichmann, Heinz, Fuhrer, Hannah, Berger, Benjamin, Zinke, Jan, Alberty, Anke, Kleiter, Ingo, Schneider-Gold, Christiane, Roth, Christian, Dunkel, Juliane, Steinbrecher, Andreas, Thieme, Andrea, Schlachetzki, Felix, Linker, Ralf A., Angstwurm, Klemens, Meisel, Andreas and Neumann, Bernhard (2021) MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation. Journal of Neurology 268, pp. 4824-4833.

Date of publication of this fulltext: 14 May 2021 05:47
Article
DOI to cite this document: 10.5283/epub.45681


Abstract

Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in similar to 6% of MG, but data on outcome of MuSK-MCs are still lacking. We made a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody positive MG (AchR-MG) or MuSK-MG between 2006 and 2015 in a ...

Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in similar to 6% of MG, but data on outcome of MuSK-MCs are still lacking. We made a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody positive MG (AchR-MG) or MuSK-MG between 2006 and 2015 in a retrospective German multicenter study. We identified 19 MuSK-AB associated MCs in 15 patients and 161 MCs in 144 patients with AchR-ABs only. In contrast to patients with AchR-AB, MuSK-AB patients were more often female (p = 0.05, OR = 2.74) and classified as Myasthenia Gravis Foundation of America-class IV before crisis (p = 0.04, OR = 3.25). MuSK-AB patients suffer more often from multiple chronic disease (p = 0.016, OR = 4.87) and were treated more invasively in terms of plasma exchanging therapies (not significant). The number of days of mechanical ventilation (MV) (43.0 +/- 53.1 vs. 17.4 +/- 18; p < 0.0001), days on an intensive care unit (ICU) (45.3 +/- 49.5 vs. 21.2 +/- 19.7; p < 0.0001), and hospital-length of stay (LOS) (55.9 +/- 47.6 vs. 28.8 +/- 20.9 days; p < 0.0001) were significantly increased in MuSK-MC. Remarkable is that these changes were mainly due to patients with MusK-ABs only, whereas patients' outcome with both antibodies was similar to AchR-MCs. Furthermore, our data showed a shortened duration of MV after treatment with plasma exchanging therapies compared to treatment with intravenous immunoglobulin in MuSK-MCs. We conclude that MuSK-AB-status is associated with a longer need of MV, ICU-LOS, and hospital-LOS in MC, and therefore recommend early initiation of a disease-specific therapy.



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Details

Item typeArticle
Journal or Publication TitleJournal of Neurology
Publisher:SPRINGER HEIDELBERG
Open Access Type:DEAL (Springer)
Place of Publication:HEIDELBERG
Volume:268
Page Range:pp. 4824-4833
Date10 May 2021
InstitutionsMedicine > Lehrstuhl für Neurologie
Identification Number
ValueType
10.1007/s00415-021-10603-9DOI
KeywordsGRAVIS; AUTOANTIBODIES; MORTALITY; Myasthenia gravis; Myasthenic crisis; Autoimmune diseases; Antibody status; MuSK-antibodies; Outcome
Dewey Decimal Classification600 Technology > 610 Medical sciences Medicine
StatusPublished
RefereedYes, this version has been refereed
Created at the University of RegensburgPartially
URN of the UB Regensburgurn:nbn:de:bvb:355-epub-456817
Item ID45681

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